Textbook of Hemophilia, Second Edition

Textbook of Hemophilia, Second Edition......Page 5
Contents......Page 7
Contributors......Page 9
Historical introduction......Page 15
1: Overview of hemostasis......Page 21
2: Cellular processing of factor VIII and factor IX......Page 27
3: Molecular basis of hemophilia A......Page 38
4: Phenotypic–genotypic relationship......Page 47
5: Prophylaxis......Page 52
6: Continuous infusion of coagulation products in hemophilia......Page 58
7: Inhibitors to factor VIII—immunology......Page 66
8: Genetic and environmental risk factors for inhibitor development......Page 71
9: Inhibitors to factor VIII: mild and moderate hemophilia......Page 76
10: Inhibitors to factor VIII/IX: immune tolerance......Page 80
11: Inhibitors to factor VIII: treatment of acute bleeds......Page 89
12: Acquired inhibitors to factor VIII......Page 95
13: Hemophilia B—molecular basis......Page 102
14: Factor IX inhibitors in hemophilia B......Page 108
15: Treatment of inhibitors in hemophilia B......Page 111
16: Pharmacokinetics......Page 118
17: The neonate with hemophilia......Page 125
18: Work-up of a bleeding child......Page 132
19: Care of the child with hemophilia......Page 141
20: Adolescence......Page 147
21: Hemophilia and medicine in old age......Page 152
22: Products used to treat hemophilia: recombinant products......Page 160
23: Plasma-derived coagulation factor concentrates......Page 171
24: Products used to treat hemophilia: recombinant factor VIIa......Page 177
25: Products used to treat hemophilia: dosing......Page 183
26: Products used to treat hemophilia: regulation......Page 188
27: Joint replacement......Page 194
28: Synoviorthesis in hemophilia......Page 200
29: Pseudotumors in patients with hemophilia......Page 205
30: Imaging modalities for assessment of hemophilic arthropathy......Page 209
31: Physiotherapy in the management of hemophilia......Page 218
32: Clinimetric instruments in hemophilia......Page 225
33: Hepatitis and hemophilia......Page 233
34: Transfusion-transmitted disease: emerging infections......Page 240
35: Hemophilia gene therapy: an overview......Page 244
36: Gene therapy trials in hemophilia A and B......Page 249
37: Gene therapy: molecular engineering of factor VIII and factor IX......Page 257
38: Laboratory assays in hemophilia......Page 265
39: Standardization of assays in hemophilia......Page 272
40: Global laboratory assays in hemophilia......Page 281
41: Obstetrics and gynecology: hemophilia......Page 287
42: von Willebrand disease: molecular aspects......Page 296
43: von Willebrand disease: epidemiology......Page 304
44: von Willebrand disease: biologic diagnosis......Page 312
45: Classification and clinical aspects of von Willebrand disease......Page 320
46: Women and von Willebrand disease......Page 327
47: Treatment of von Willebrand disease: desmopressin......Page 334
48: Treatment of von Willebrand disease: therapeutic concentrates......Page 338
49: Factor II......Page 346
50: Factor V and combined factor V and VIII deficiencies......Page 350
51: Congenital factor VII deficiency......Page 359
52: Factor X and factor X deficiency......Page 366
53: Factor XI deficiency......Page 373
54: Factor XIII deficiency......Page 380
55: Fibrinogen......Page 387
56: Miscellaneous bleeding disorders......Page 394
57: Emergency management of hemophilia......Page 412
58: Quality of life in hemophilia......Page 419
59: The economics of hemophilia treatment......Page 427
60: National hemophilia databases......Page 433
61: Comprehensive care and delivery of care: the developed world......Page 438
62: Comprehensive care and delivery of care in hemophilia: the developing world......Page 447
63: Comprehensive care and delivery of care: the global perspective......Page 453
Index......Page 461
colour plates......Page 163